Potential clinical impact of compounded versus noncompounded intrathecal baclofen.

OBJECTIVE: To assess the differences between commercial and pharmacy-compounded preparations of baclofen for intrathecal administration. DESIGN: Random sample. SETTING: Pharmacies in the United States advertising compounded intrathecal baclofen preparation. PARTICIPANTS: Not applicable. INTERVENTIONS: Intrathecal baclofen (ITB) samples were collected from 1 Food and Drug Administration-approved commercial source and 6 compounding pharmacies. An independent analysis of drug concentration and density was conducted. Information regarding ordering process, manufacturing, packaging, storage, and expiration was collected. MAIN OUTCOME MEASURE: Comparison of concentration and density variations. RESULTS: Twenty-nine ITB samples in concentrations of 2000, 3000, 4000, 5000, and 6000 microg/mL were analyzed. Over 40% of compounded samples were more than 5% above or below labeled concentration. Twenty-two percent of compounded samples were more than 10% above or below labeled concentration. The only samples with no concentration deviation and consistent drug density were the commercially available, noncompounded products. CONCLUSIONS: Compounding pharmacies have variable practices in the provision of ITB. A high incidence of concentration inaccuracy existed. The use of compounded ITB may result in unintended dose alterations. Variable clinical efficacy, or life-threatening overdose or withdrawal may occur in patients who are sensitive to slight dose fluctuations. Given the variability of these compounded ITB samples, informed consent to use these products and understanding of potential side effects should be reviewed with patients.

Pediatric rehabilitation: 2. Environmental factors affecting participation.

OBJECTIVE: This self-directed learning module highlights the environmental factors that influence the participation of children and youth with disabilities in life situations, including activities of self-care, mobility, socialization, education, recreation, and community life. It is part of the study guide on pediatric rehabilitation in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation and pediatric medicine. This module emphasizes the participation of children with disabilities in adapted sports and reviews mobility devices to promote function, in the context of the International Classification of Functioning and in reference to the Americans with Disabilities Act. It provides the physiatrist with strategies to promote community participation, functional independence and healthcare transitions for children with disabilities as they approach adulthood. The goal of this article is to improve the learner's treatment strategies to maximize the participation of children and youth with disabilities in all settings, particularly schools and communities.

Pediatric rehabilitation: 3. Facilitating family-centered treatment decisions.

OBJECTIVE: This self-directed learning module focuses on the role of accurate diagnosis, psychological support, and family integration of children who have chronic impairments such as pain, spasticity, or cognitive disability. It is part of the study guide on pediatric rehabilitation in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation and pediatric medicine. The role of therapeutic, medical (traditional and nontraditional) and psychological interventions that improve family and individual function are emphasized. The goal of this article is to refine a learner's knowledge of the impact family-centered care can have on the medical, psychological, financial, and functional capabilities of families to improve treatment decisions in the context of children with disability.

Pediatric rehabilitation: 4. Prescribing assistive technology to promote community integration.

OBJECTIVE: This self-directed learning module highlights the equipment and assistive technology needs of children and youth with disabilities. This article specifically focuses on preparing families and patients for equipment transitions that occur over the course of childhood and adolescence including progressing from stroller to wheelchair to powerchair, as well as job training and use of augmentative communication. It is part of the study guide on pediatric rehabilitation in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation and pediatric medicine. The goal of this article is to modify the learner's current practice techniques to ensure that assistive technology is used to promote community integration from early childhood through transition and into adulthood.

Pediatric rehabilitation: 1. Common medical conditions in children with disabilities.

OBJECTIVE: This self-directed learning module focuses on the physiatric management of the common morbidities associated with pediatric traumatic brain injury and cerebral palsy. It is part of the study guide on pediatric rehabilitation in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation and pediatric medicine. The goal of this article is to enhance the learner's knowledge regarding current physiatric management of complications related with pediatric traumatic brain injury and cerebral palsy.

Pediatric rehabilitation: 5. Transitioning teens with disabilities into adulthood.

OBJECTIVE: This self-directed learning module focuses on preparing adolescent patients with special health care needs for adulthood by promoting their independence in their own self-care; helping them to navigate issues of sexuality, marriage, and parenting; preparing the patient and family to make guardianship decisions during the transition between childhood and adulthood; and planning for higher education or vocation. Emphasis will be on the role of the physiatrist in providing this guidance and its importance in improving the patient's quality of life. It is part of the study guide on pediatric rehabilitation in the Self-Directed Physiatric Education Program for practitioners and trainees in physical medicine and rehabilitation and pediatric medicine. The goal of this article is to refine the learner's knowledge of preparing adolescent patients with special health care needs for adulthood to improve their quality of life.

Adapted recreational and sports programs for children with disabilities: A decade of experience.

OBJECTIVE: To identify and describe community based adapted sports and recreational programs (SARPs) for children with physically disabilities, documenting program types, benefits, challenges, growth and/or decline, and lessons they have learned over a 10-year period. METHODS: In 1996, a total of 277 children's hospitals and freestanding rehabilitation hospitals stating that they provided pediatric rehabilitation services were contacted and asked to provide information regarding adapted recreational and sports programs in their region. Seventy-nine SARPs were identified, contacted, and survyed about programming, benefits and challenges they faced. They were then re-surveyed in 2006 for comparison data. RESULTS: Ten years ago, the average SARP served 25 or fewer clients and was led by a therapeutic recreation specialist with assistance from volunteers. Most programs had been in place for 5 years or more, met weekly for 2-3 hours, and were recreational in orientation. Activities varied, with basketball, aquatics, horseback riding and snow skiing being most common. Fund-raisers and grants supported most programs, and securing funding was their greatest challenge. Participant benefits noted by programs included improved socialization, enhanced physical fitness, increased self esteem, improved therapeutic skills (ADL's, transfers, etc.), enhanced cognition, expanded client independence, improved community relations, and enhanced leisure skills. Ten years later, the majority of SARPs noted similar benefits, and reported an increase in number of participants despite continued challenges with funding and staffing. Leadership and mentorship by those with disabilities was still very low, but community awareness of the abilities of those with disabilities had increased. CONCLUSIONS: Adapted sports and recreation programs surveyed in 1996 and again in 2006, report overall that their health is good, and many have retained the same programming, financial support mechanisms, leadership and participant mix over the years. Many have increased athlete participation despite financial challenges being a predominant concern. They report that their staying power is dependent on many factors, including leadership, participant referrals, an ample supply of volunteers, and consistent community and financial support. They feel that their success is important to the physical and psychological well-being of individuals with disabilities and that an increased focus on corporate sponsorship, participation and mentorship by those with disabilities may assist with future growth. Increased school involvement, development of more competitive vs. recreational programs, and improved research documenting the physical and psychological benefits of adapted sports programs were also recommended to improve future SARP stability.

Cerebral palsy masking spinal muscular atrophy.

Spinal muscular atrophy (SMA) is an autosomal recessive anterior horn cell disease that results in progressive muscular weakness and atrophy without sensory involvement. A wide clinical spectrum that ranges from early death to essentially normal adult live exists. We describe a case of two 12 years olds, who represent two of three surviving non-identical quadruplets, born at 25 weeks gestational age. A diagnosis of hypotonic cerebral palsy (CP) was made in early childhood and early intervention services were initiated. At 3 years of age, MRI's showed white matter changes. Both briefly attained Gross Motor Functional Classification Scale (GMFCS) 3 status, but by 12 years of age their ambulatory abilities had decreased to Level 4. Physical Medicine and Rehabilitation (PM&R) physicians were consulted. On exam, distal lower extremities atrophy, hypotonia, hyporeflexia, and muscle weakness were noted. Neither child had upper motor neuron signs or spasticity. Cognition was normal. Neuromuscular disorder was suspected and genetic testing confirmed spinal muscular atrophy in both patients. While prior MRI/CT demonstrated static encephalopathy, recognition of symptoms and signs consistent with neuromuscular disease should have led to a secondary diagnosis. Therapeutic and surgical treatment decisions may have differed. Fragmentation of care and lack of a comprehensive team approach also contributed to the delay in recognition of their dual diagnosis.